Glucagonoma natural history, complications and prognosis

If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia. The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Age, grade, and distant metastases are the most significant predictors of survival.

• If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia.
• Glucagonoma has a very slow growth rate compared to most malignant tumors.

Complications of glucagonoma include:

• Metastasis:
  • Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis.
  • Metastasis occurs mainly in the liver but in few cases, it can occur in lymph nodes, peritoneum, lung, and adrenals.
• Weight loss: due to the catabolic effect of glucagon, most patients lose weight.
• Anemia
• Neuropsychiatric manifestations include depression, dementia, psychosis, and agitation
• Reversible dilated cardiomyopathy^[1]

Prognosis of glucagonoma depends on the following:

• Whether or not the tumor can be removed by surgery.
• The stage of the tumor, the size of the tumor, whether cancer has spread outside the pancreas.
• The patient’s general health.
• Whether the tumor has just been diagnosed or has recurred.
• The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma.
• The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis.

Additionally:

• Age, grade, and distant metastases are the most significant predictors of survival.
• Five and 10-year survival rates for patients undergoing resection of gastroenteropancreatic neuroendocrine tumors.^[2]
• Sixty percent of glucagonomas are malignant. Once the tumor is metastatic, the cure is rare.^[3]

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